POS0755 TOWARDS THE DEVELOPMENT OF COMPOSITE PARENT-CENTERED DISEASE ACTIVITY SCORES FOR JUVENILE DERMATOMYOSITIS
Authors: S. Rosina, A. I. Rebollo Giménez, L. Tarantola, R. Naddei, A. Consolaro, A. Pistorio, A. Ravelli
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> In recent years, increasing attention has been paid to the development of parent- and child-centered composite disease activity scores for the assessment of health status of children with rheumatic diseases. <h3>Objectives</h3> This study is aimed to develop and test an entirely parent-centered composite disease activity score for JDM, named parent Juvenile DermatoMyositis Activity Index (parJDMAI). Two versions of the score, which differed in the fourth item included, were evaluated. <h3>Methods</h3> Both parJDMAI1 and parJDMAI2 include the following items: 1) parent assessment of skin disease activity (Parent Skin Scale) on a 0-5 scale, by giving 1 point to the presence of each of the following: i) rash on eyelids, ii) nose/cheeks, iii) knuckles, iv) trunk/arms, v) skin ulceration; 2) parent assessment of muscle disease activity (Parent Muscle Scale) on a 0-5 scale, by giving 1 point to the presence of each of the following: i) fatigue/discomfort, ii) muscle weakness, iii) muscle pain, iv) voice change, v) difficulty swallowing; 3) parent assessment of child's fatigue on a 0-10 visual analog scale (VAS) (0 = no fatigue; 10 = maximum fatigue). As fourth item, the parJDMAI1 includes the parent global assessment of child's wellbeing, measured on a 0-10 VAS (0 = best; 10 = worst), whereas the parJDMAI2 includes the parent global assessment of disease activity, measured on a 0-10 VAS (0 = no activity; 10 = maximum activity). To give the four components of the tools the same weight, the scores of the Parent Skin and Muscle Scales were doubled. Thus, the total score of both instruments ranges from 0 to 40. Initial validation was conducted on a multicentric sample of 213 patients followed in standard clinical care and evaluated prospectively (number of visits = 577), and on a monocentric sample including 50 patients, all assessed at baseline and 32 also assessed after a median of 3.9 months (number of visits = 82). Validation analyses included calculation of the correlations between individual parJDMAI items and physician-centered JDM outcome measures, and between the total score of parJDMAI1 and parJDMAI2 with that of the global composite disease activity scores for JDM named JDMAI1 and JDMAI2. Because both JDMAI1 and JDMAI2 include the parent global assessment of child's wellbeing, which is also part of parJDMAI1, we also tested the correlations of parJDMAI1 with reduced versions of JDMAI1 and JDMAI2, that included only the 3 physician-centered items. Spearman correlations were defined as low, moderate or high when r<sub>S</sub> was &lt; 0.4, ≥ 0.4 and ≤ 0.7, or &gt; 0.7, respectively. <h3>Results</h3> Correlations between individual components of parJDMAI1 and parJDMAI2 and physician-centered JDM outcome measures were low-to-moderate in the multicentric sample, but moderate in the monocentric sample. Likewise, correlations between the scores of parJDMAI1 and parJDMAI2 and that of original and reduced versions of JDMAI1 and JDMAI2 were strong in the monocentric sample, but moderate in the multicentric sample. <h3>Conclusion</h3> The new parent-centered composite disease activity scores revealed satisfactory measurement properties. That correlations with physician-centered outcome measures and original and reduced JDMAI versions were higher in the monocentric sample than in the multicentric cohort indicates that the proposed tools should be further tested in different clinical and cultural environments before their widespread use can be recommended. <h3>Reference</h3> [1]Rosina et al. Rheumatology 2019; 58:1196-205 <h3>Acknowledgements</h3> This work was partially supported by the Fundación Española de Reumatología (FER). <h3>Disclosure of Interests</h3> None Declared.